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Köhlmeier-Degos disease is an interferonopathy characterized by type I and II interferon-driven inflammatory vasculopathy
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DOI：10.1016/j.xcrm.2026.102947.png)
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Abstract
En 中文
• Degos disease shows type I and II interferon activation across tissues • Degos disease shows an interferon-γ bias versus systemic lupus erythematosus • Cytotoxic T cells show activation and clonal restriction • Ruxolitinib in one patient is associated with reduced interferon programs
Keywords:
Köhlmeier-Degos disease
Degos disease
single-cell RNA sequencing
skin
central nervous system
gastrointestinal tract
interferonopathy
vasculopathy
JAK-STAT signaling
IFN-γ
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